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anti-HMBS antibody :: Rabbit HMBS Polyclonal Antibody

Scan QR to view Datasheet Catalog #    MBS9407926
Unit / Price
0.1 mL  /  $255 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

HMBS, Polyclonal Antibody

★Popular Item★
 Also Known As   

HMBS Antibody

 Product Synonym Names    UPS; PBGD; PORC; PBG-D
 Product Gene Name   

anti-HMBS antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 OMIM    176000
 3D Structure    ModBase 3D Structure for P08397
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 Clonality    Polyclonal
 Host    Rabbit
 Species Reactivity    Human, Mouse, Rat
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 Specificity    The antibody detects endogenous level of total HMBS protein.
 Purity/Purification    Antibodies were purified by affinity purification using immunogen.
 Form/Format    Supplied at 1.0mg/mL in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
 Concentration    1.0 mg/ml (lot specific)
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 Immunogen Type    Recombinant Protein
 Immunogen Description    Recombinant protein of human HMBS.
 Target Name    HMBS
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 Preparation and Storage    Store at -20 degree C
 Other Notes    Small volumes of anti-HMBS antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Related Product Information for anti-HMBS antibody

   This gene encodes a member of the hydroxymethylbilane synthase superfamily. The encoded protein is the third enzyme of the heme biosynthetic pathway and catalyzes the head to tail condensation of four porphobilinogen molecules into the linear hydroxymethylbilane. Mutations in this gene are associated with the autosomal dominant disease acute intermittent porphyria. Alternatively spliced transcript variants encoding different isoforms have been described.
 Product Categories/Family for anti-HMBS antibody    Total protein Ab
 Applications Tested/Suitable for anti-HMBS antibody   

Western Blot (WB), Immunohistochemistry (IHC)

 Application Notes for anti-HMBS antibody    Western blotting: 1:500 - 1:2000
Immunohistochemistry: 1:50 - 1:100
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 Western blot of anti-HMBS antibody    Western blot analysis of extracts of various cell lines, usingHMBS antibody.
anti-HMBS antibody Western blot image
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 Immunohistochemical of anti-HMBS antibody    Immunohistochemical analysis of paraffin-embedded humanstomach using HMBS antibody at dilution of 1:100 (200x lens).
anti-HMBS antibody Immunohistochemical image
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 Immunohistochemical of anti-HMBS antibody    Immunohistochemical analysis of paraffin-embedded human stomach cancer using HMBS antibody at dilution of 1:100 (200x lens).
anti-HMBS antibody Immunohistochemical image
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NCBI/Uniprot data below describe general gene information for HMBS. It may not necessarily be applicable to this product.
 NCBI GI #    20149500
 NCBI GeneID    3145
 NCBI Accession #    NP_000181.2 [Other Products]
 NCBI GenBank Nucleotide #    NM_000190.3 [Other Products]
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 UniProt Primary Accession #    P08397 [Other Products]
 UniProt Secondary Accession #    P08396; Q16012; A8K2L0; G3V1P4; G5EA58 [Other Products]
 UniProt Related Accession #    P08397 [Other Products]
 Molecular Weight    39kD
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 NCBI Official Full Name    porphobilinogen deaminase isoform 1
 NCBI Official Synonym Full Names    hydroxymethylbilane synthase
 NCBI Official Symbol    HMBS [Similar Products]
 NCBI Official Synonym Symbols   
UPS; PBGD; PORC; PBG-D
[Similar Products]
 NCBI Protein Information    porphobilinogen deaminase
 UniProt Protein Name    Porphobilinogen deaminase
 UniProt Synonym Protein Names   
Hydroxymethylbilane synthase; HMBS; Pre-uroporphyrinogen synthase
 UniProt Gene Name    HMBS [Similar Products]
 UniProt Synonym Gene Names    PBGD; UPS; PBG-D; HMBS [Similar Products]
 UniProt Entry Name    HEM3_HUMAN
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 NCBI Summary for HMBS    This gene encodes a member of the hydroxymethylbilane synthase superfamily. The encoded protein is the third enzyme of the heme biosynthetic pathway and catalyzes the head to tail condensation of four porphobilinogen molecules into the linear hydroxymethylbilane. Mutations in this gene are associated with the autosomal dominant disease acute intermittent porphyria. Alternatively spliced transcript variants encoding different isoforms have been described. [provided by RefSeq, Jul 2008]
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 UniProt Comments for HMBS    HMBS: Tetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps. Defects in HMBS are the cause of acute intermittent porphyria (AIP). AIP is a form of porphyria. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. AIP is an autosomal dominant form of hepatic porphyria characterized by acute attacks of neurological dysfunctions with abdominal pain, hypertension, tachycardia, and peripheral neuropathy. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors. Belongs to the HMBS family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Transferase; Mitochondrial; Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; EC 2.5.1.61

Chromosomal Location of Human Ortholog: 11q23.3

Cellular Component: cytosol

Molecular Function: hydroxymethylbilane synthase activity

Biological Process: porphyrin metabolic process; peptidyl-pyrromethane cofactor linkage; protoporphyrinogen IX biosynthetic process; heme biosynthetic process

Disease: Porphyria, Acute Intermittent
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 Research Articles on HMBS    1. we report a novel PBGD missense mutation.
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with anti-HMBS antibodyPathways associated with anti-HMBS antibody
 Reference Product  PubMed Publications
 HPRT1 antibody  >34 publications with HMBS and HPRT1
 SDHA antibody  >24 publications with HMBS and SDHA
 ALAS1 antibody  >5 publications with HMBS and ALAS1
 FECH antibody  >3 publications with HMBS and FECH
 CPOX antibody  >3 publications with HMBS and CPOX
 UROD antibody  >2 publications with HMBS and UROD
 UROS antibody  >1 publications with HMBS and UROS
 ALAD antibody  >1 publications with HMBS and ALAD
 Products by Pathway  Pathway Diagram
 Heme Biosynthesis Pathway antibodies  Heme Biosynthesis Pathway Diagram
 Heme Biosynthesis Pathway antibodies  Heme Biosynthesis Pathway Diagram
 Metabolic Pathways antibodies  Metabolic Pathways Diagram
 Metabolism Pathway antibodies  Metabolism Pathway Diagram
 Metabolism Of Porphyrins Pathway antibodies  Metabolism Of Porphyrins Pathway Diagram
 Porphyrin And Chlorophyll Metabolism Pathway antibodies  Porphyrin And Chlorophyll Metabolism Pathway Diagram
 Porphyrin And Chlorophyll Metabolism Pathway antibodies  Porphyrin And Chlorophyll Metabolism Pathway Diagram
 Heme Biosynthesis Pathway antibodies  Heme Biosynthesis Pathway Diagram
 Superpathway Of Heme Biosynthesis From Glycine antibodies  Superpathway Of Heme Biosynthesis From Glycine Diagram
 Tetrapyrrole Biosynthesis Pathway antibodies  Tetrapyrrole Biosynthesis Pathway Diagram
Diseases associated with anti-HMBS antibodyOrgans/Tissues associated with anti-HMBS antibody
 Disease Name  Pubmed Publications
 Porphyria, Acute Intermittent Antibodies  >61 publications with HMBS and Porphyria, Acute Intermittent
 Liver Diseases Antibodies  >59 publications with HMBS and Liver Diseases
 Inflammation Antibodies  >7 publications with HMBS and Inflammation
 Carcinoma, Hepatocellular Antibodies  >6 publications with HMBS and Carcinoma, Hepatocellular
 Liver Neoplasms Antibodies  >5 publications with HMBS and Liver Neoplasms
 Necrosis Antibodies  >5 publications with HMBS and Necrosis
 Nervous System Diseases Antibodies  >5 publications with HMBS and Nervous System Diseases
 Lung Diseases Antibodies  >5 publications with HMBS and Lung Diseases
 Kidney Diseases Antibodies  >3 publications with HMBS and Kidney Diseases
 Neoplasm Metastasis Antibodies  >3 publications with HMBS and Neoplasm Metastasis
 Organ/Tissue Name  Pubmed Publications
 Blood Antibodies  >30 publications with HMBS and Blood
 Liver Antibodies  >19 publications with HMBS and Liver
 Muscle Antibodies  >13 publications with HMBS and Muscle
 Brain Antibodies  >10 publications with HMBS and Brain
 Heart Antibodies  >8 publications with HMBS and Heart
 Lung Antibodies  >6 publications with HMBS and Lung
 Kidney Antibodies  >5 publications with HMBS and Kidney
 Ovary Antibodies  >5 publications with HMBS and Ovary
 Bone Marrow Antibodies  >4 publications with HMBS and Bone Marrow
 Bone Antibodies  >4 publications with HMBS and Bone
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